Surgical correction of total anomalous pulmonary venous connection

총폐정맥 이상환류증의 수술요

Total anomalous pulmonary venous connection [TAPVC] defines a group of congenital heart disease which have in common the entire pulmonary venous drainage returning directly or indirectly to the right atrium instead of to the left atrium. Although this disorder represents only 1.3% of cases of congenital heart disease, if untreated the resultant mortality is greater than 80% in the 1st year of life. And since there is no satisfactory palliative treatment, correction of TAPVC Is high on the list of indications for open heart surgery in the 1st year of life. This paper describes 10 patients who underwent surgical correction of this disorder at SNUH between 1978 and 1983. 1. 7 were males and 3 females, with ages ranging from 5 months to 24 years. 2. 7 were supracardiac type, 2 cardiac type, and 1 mixed type TAPVC. We didnt experience infracardiac type. 3. All showed some degree of pulmonary hypertension preoperatively. 4. In 4 cases of supracardiac type, total circulatory arrest was used in brief period during anastomosis between common pulmonary venous trunk and left atrium. In the other cases, usual cardiopulmonary bypass with moderate to deep hyperthermia was used. 5. There were 2 cases of mortality; 1 died at operation, and the other at 4 months due to congestive heart failure. 6. Mortality seemed not closely related to age, body weight, or severity of pulmonary hypertension.